Inclusion body myositis and paraproteinemia: incidence and immunopathologic correlations

Annals of Neurology
M C DalakasC Juarez

Abstract

Serum from 70 patients with sporadic inclusion body myositis (IBM) was subjected to agarose gel immunofixation electrophoresis. The IgG extracted from 9 patients with monoclonal proteins, 3 without, and 2 control subjects and was purified, biotinylated, and applied to muscle biopsy sections for immunocytochemistry and to purified muscle protein fractions for immunoblots. Sixteen of 70 (22.8%) patients with IBM, compared with 2% of age-matched controls, had a monoclonal gammopathy characterized as IgG lambda in 9 patients, IgG kappa in 4, IgM kappa in 2, and IgA lambda in 1. The mean age of IBM patients with gammopathy was 60.6 years (range, 35-77 years), compared with 66.1 years (range, 42-80 years) of the IBM patients without gammopathy. The IgG of the patients, more often than that of the control subjects, immunostained myonuclei and recognized various muscle proteins of 35 to 145 kd. We conclude that IBM, regardless of age, is frequently associated with monoclonal gammopathies, which often recognize various muscle components, especially myonuclei, suggesting disturbed immunoregulation.

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Citations

Jul 4, 2012·Journal of Neurology·Joerg-Patrick Stübgen
Aug 26, 2006·Nature Clinical Practice. Neurology·Marinos C Dalakas
Jul 23, 2009·BMJ : British Medical Journal·Steven A Greenberg
Mar 29, 2007·Arthritis Research & Therapy·Cecilia GrundtmanIngrid E Lundberg
Jun 2, 2011·PloS One·Mohammad SalajeghehSteven A Greenberg
Feb 20, 2013·The Canadian Journal of Neurological Sciences. Le Journal Canadien Des Sciences Neurologiques·Ahmad R AbuzinadahLawrence Korngut
Feb 20, 2010·Gastroenterology Research and Practice·Elke KaemmererNikolaus Gassler
Dec 20, 2015·Neurology·Marinos C Dalakas, Jens Schmidt
Mar 30, 2011·Rheumatic Diseases Clinics of North America·Guillermo E Solorzano, Lawrence H Phillips
Dec 28, 2007·Neuromuscular Disorders : NMD·M Needham, F L Mastaglia
Jun 22, 2007·Lancet Neurology·Merrilee Needham, Frank L Mastaglia
Mar 6, 2013·Annals of Neurology·Helma PlukGer J M Pruijn
Aug 16, 2000·Archives of Physical Medicine and Rehabilitation·A J Boon, K A Stolp-Smith
Jun 20, 2015·Neurology. Neuroimmunology and Neuroinflammation·Ali AlshehriAlan Pestronk
Dec 17, 2014·Journal of Clinical Neuroscience : Official Journal of the Neurosurgical Society of Australasia·Frank L Mastaglia, Merrilee Needham
Mar 28, 2003·Muscle & Nerve·Frank L MastagliaPaul J Zilko
Jul 11, 2006·Muscle & Nerve·Steven A GreenbergAnthony A Amato
Jul 9, 2003·Immunological Reviews·Robert A Kyle, S Vincent Rajkumar
Oct 22, 2013·Expert Review of Clinical Immunology·Jens Schmidt, Marinos C Dalakas
Jun 8, 2017·Annals of Clinical and Translational Neurology·Christian W KellerJan D Lünemann
Dec 22, 2006·The Journal of Immunology : Official Journal of the American Association of Immunologists·Elizabeth M BradshawKevin C O'Connor
Mar 20, 2019·Journal of General Internal Medicine·Jason HomUNKNOWN Undiagnosed Diseases Network
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Nov 13, 2021·Critical Reviews in Clinical Laboratory Sciences·Konstantinos I TsamisIoannis Sarmas

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